Temporal Trends in Acute Adrenal Insufficiency Events in Children With Congenital Adrenal Hyperplasia During 2019-2022.

Tseretopoulou, X.; Ali, SR.; Bryce, J.; Amin, N.; Atapattu, N.; Bachega, TASS.; Baronio, F.; Ortolano, R.; Birkebaek, NH.; Bonfig, W.; Cools, M.; Davies, JH.; Thomas, T.; de Vries, L.; Elsedfy, H.; Amr, NH.; Flueck, CE.; Globa, E.; Guran, T.; Yavas-Abali, Z.; Guven, A.; Hannema, SE.; Iotova, V.; Konrad, D.; Lenherr-Taube, N.; Krone, NP.; Leka-Emiri, S.; Vlachopapadopoulou, E.; Lichiardopol, C.; Marginean, O.; Markosyan, R.; Neumann, U.; Niedziela, M.; Banaszak-Ziemska, M.; Phan-Hug, F.; Poyrazoglu, S.; Probst-Scheidegger, U.; Randell, T.; Russo, G.; Salerno, M.; Seneviratne, S.; Shnorhavorian, M.; Thankamony, A.; Tadokoro-Curraro, R.; van den Akker, E.; van Eck, J.; Vieites, A.; Wasniewska, M.; Ahmed, SF.

Temporal Trends in Acute Adrenal Insufficiency Events in Children With Congenital Adrenal Hyperplasia During 2019-2022.

All Authors

Tseretopoulou, X.

Ali, SR.

Bryce, J.

Amin, N.

Atapattu, N.

Bachega, TASS.

Baronio, F.

Ortolano, R.

Birkebaek, NH.

Bonfig, W.

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LTHT Author

Amin, Nadia

LTHT Department

Leeds Children's Hospital
Children's Endocrinology

Publication Date

2024

Item Type

Journal Article

Abstract

Background: It is unclear whether targeted monitoring of acute adrenal insufficiency (AI) related adverse events (AE) such as sick day episodes (SDEs) and hospitalization rate in congenital adrenal hyperplasia (CAH) is associated with a change in the occurrence of these events. Aim: Study temporal trends of AI related AE in the I-CAH Registry. Methods: In 2022, data on the occurrence of AI-related AE in children aged <18 years with 21-hydroxylase deficiency CAH were compared to data collected in 2019. Results: In 2022, a total of 513 children from 38 centers in 21 countries with a median of 8 children (range 1-58) per center had 2470 visits evaluated over a 3-year period (2019-2022). The median SDE per patient year in 2022 was 0 (0-2.5) compared to 0.3 (0-6) in 2019 (P = .01). Despite adjustment for age, CAH phenotype and duration of study period, a difference in SDE rate was still apparent between the 2 cohorts. Of the 38 centers in the 2022 cohort, 21 had also participated in 2019 and a reduction in SDE rate was noted in 13 (62%), an increase was noted in 3 (14%), and in 5 (24%) the rate remained the same. Of the 474 SDEs reported in the 2022 cohort, 103 (22%) led to hospitalization compared to 299 of 1099 SDEs (27%) in the 2019 cohort (P = .02). Conclusion: The I-CAH Registry can be used for targeted monitoring of important clinical benchmarks in CAH. However, changes in reported benchmarks need careful interpretation and longer-term monitoring.